Improving life expectancy of patients with scleroderma: results from the South Australian Scleroderma Register

Background: Scleroderma is a rare connective tissue disorder characterised by inflammation, vasculopathy and excessive fibrosis. Patients with scleroderma are known to have decreased life expectancy.

Aim: To investigate changes in life expectancy in patients with scleroderma over a 30-year period.

Methods: Utilising the South Australian Scleroderma Register, deceased patients were identified. We examined changes in age of death and duration of disease in these patients over three time periods: 1985–1994, 1995–2004 and 2005–2015. Analyses of scleroderma subtypes were performed, and comparisons were made to the general South Australian population.

Results: A total of 413 deceased patients was identified. Females were overrepresented 315 to 98; 265 had limited scleroderma, 90 diffuse and 22 overlap disease. Over 30 years, the mean age of death improved from 66.4 to 74.5 years (P < 0.001). Duration of disease improved from 12.1 to 22.9 years (P < 0.001). Improvement in survival was seen in limited (P = 0.001), diffuse (P = 0.04) and overlap (P = 0.04) subgroups. The increase in survival was only seen for female (9.8 ± 4.2 years) but not male (1.4 ± 6.7 years) patients.

Conclusion: Over the last 30 years, survival has significantly improved for female but not male patients. As no disease-modifying drugs have consistently been shown to alter disease course, this improvement is likely attributable to general improvements in medical care, including that of scleroderma-related complications. While the life expectancy for limited disease is now close to that of the general population, patients with diffuse and overlap disease continue to suffer from significant early mortality.